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Original Article

Liver Function in Asymptomatic Adult Individuals with Severe α1-Antitrypsin Deficiency (Pi Z)

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Pages 543-546 | Received 31 Dec 1976, Accepted 14 Mar 1977, Published online: 23 Feb 2010
 

Abstract

Eleven adult individuals (aged 24 to 66 years) with severe χ1-antitrypsin deficiency, Pi Z. and with no clinical signs of liver disease, were investigated with a broad spectrum of liver function tests. Except for low χ1-antitrypsin levels, no evidence of abnormal hepatic protein synthesis was found. Neither could any biochemical signs of liver cell necrosis or increased liver cell regeneration be disclosed. In spite of the well-known pronounced distension of hepatic endoplasmic reticulum (E.R.) by aggregated asialo-χ1-antitrypsin in this disease, the function of the E.R. tested with the aminopyrine breath test was not found to be impaired.

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